Background: Pancreatic neuroendocrine tumors (NETs) are rare; representing less than 10% of all
primary pancreatic tumors. The minority of these tumors are functional producing a
clinical syndrome according to the hormone secreted. ACTH production by pancreatic
NETs is rare and even rarer, it occurs from liver metastasis later in the course of
the disease. Case Report: We report a case of a 30-year-old Libyan man who presented with Cushing's syndrome
due to ectopic ACTH production from liver metastases of a pancreatic NET. The pancreatic
NET was diagnosed 7 years previously when it was considered a nonfunctional at the
time of diagnosis; the tumor was resected completely then. In the current presentation
there was no evidence of the primary tumor but multiple liver metastases were present.
Conclusions: This report highlights the fact that initially non-functional pancreatic NETs may
present later with a functional hormonal syndrome either from the primary tumor itself
or from its metastatic secondary tumors elsewhere.
